Infantile systemic juvenile xanthogranuloma case with massive liver infiltration.

Leukemia cells invading the liver express liver chemokine receptors and possess characteristics of leukemia stem cells in mice with MPD-like myeloid leukemia

JXG is a benign disorder of early childhood and primarily occurs in infants and children <2 years of age. It is characterized by solitary or multiple yellow-red nodules on the skin and, rarely, in other organs. The clinical diagnosis of solitary cutaneous JXG usually requires confirmation by biopsy. Referral to a dermatologist should be obtained to exclude other disorders if multiple lesions are present. Clinicians should rule out ocular lesions with referral to an ophthalmologist if the patient is <2 years of age and has multiple lesions of JXG. If ophthalmologic consultation reveals no ocular lesions, screening every 6 months for the first 2 years of life is advised.

Objective To analyze the correlative factors of neonatal deaths in neonatology department to improve the treatment level and reduce the death rate of the hospitalized newborn patients. Method The medical records and death review data of the infants who died in the neonatology department of the Hospital from 2009 to 2015 were retrospectively reviewed. They were assigned to three groups according to the therapeutic measures before their death, including comfort care group, do not resuscitate (DNR) group and active treatment group. Gestational age, birth weight, postnatal age at death and the direct cause of death were compared between groups by χ2 test. Result A total of 337 infants were enrolled in this study. There were 112 cases (33.2%) in the comfort care group, 73 cases (21.7%) in the DNR group and 152 cases (45.1%) in the active treatment group. Compared to the proportion of the different group during 2009-2012, the proportion of the comfort care group during 2013-2015 significantly increased(38.9% vs.27.2%, P=0.023), and the proportion of the active treatment group significantly decreased (38.3% vs.52.5%, P=0.009), while the proportion of the DNR group showed no significant difference (22.9% vs.20.4%, P=0.580). The proportion of the comfort care group was significantly higher in the group of neonates who died in early neonatal period than that in the late neonatal period and post-neonatal period (42.9% vs.9.3%, P<0.001), while the proportion of the active treatment group and DNR group decreased(17.9% vs.30.9%, P=0.009; 39.2% vs.59.8%, P=0.001). The proportion the comfort care group increased with the decreasing gestational age and birth weight (P=0.001 and 0.002). Of the 34 term infants, the top two principle causes of death were severe asphyxia (41.1%, 14 cases) and congenital defects (32.4%, 11 cases). Of the 303 preterm infants, the top three direct causes of death were neonatal respiratory distress syndrome (31.4%, 95 cases), neonatal pulmonary hemorrhage (22.4%, 68 cases) and congenital defects (6.3%, 19 cases). Conclusion The proportion of comfort care and DNR was higher than the proportion of active treatment before the newborns′ death and it was relative to gestational age and birth weight. Preterm infants were the predominant population of the infant deaths in the neonatology department. Neonatal respiratory distress syndrome was the principle cause of death in preterm infants, and severe asphyxia was the main cause of death in term infants. Key words: Cause to death; Asphyxia neonatorum; Congenital abnormalities; Respiratory distress syndrome, newborn; Pulmonary hemorrhage

Sarcoidosis involves the liver in approximately 65% of all patients, but laboratory evidence of liver disease is usually minimal and clinical manifestations rare. In two patients with massive liver infiltration, both followed closely with serial liver function tests and percutaneous biopsies, one died after repeated hemorrhage from esophageal varices 3 years after diagnosis, while the other, still living, has developed no evidence of portal hypertension after 4 years, although her liver changes were fully as severe. Both were treated with steroids. The mechanisms of the portal hypertension is still in doubt, although there is some evidence that it is due to presinusoidal block, without the classic pathologic changes of cirrhosis.

Pancreatic head tumor in an infant with new-onset jaundice.

Firm, enlarging papule on an infant's proximal thigh

Objective To review the direct causes of death from breast cancer in Colombia according to data from death certificates in 2008. Material and Methods We reviewed the direct causes of death in patients whose code of underlying cause of death was malignant tumor of the breast according to the National Statistics Department of Colombia's (DANE from Spanish initials) records of death in 2008. Distribution is evaluated by diagnostic code, age, educational level, marital status, social security and place of death. Results In women, the main direct causes of death were respiratory insufficiency or failure, cardiac arrest, multiple or multisystem organ failure, breast cancer and metastatic breast cancer. Most of the death codes were C509 and they were issued for women over 50 years. In men, the most common cause was respiratory failure. Conclusions The main denominations listed on death records as a direct cause of death from breast cancer were respiratory failure and arrest and cardiopulmonary arrest. However, it is evident that this arises from problems in the registry that are the result of not following the International Classification of Diseases (ICD-10). Improving the quality of death records is required so that all the information given by the death certificates can be used more effectively.

Massive Infiltration of Liver by Metastatic Adenocarcinoma: A Rare Cause of Acute Hepatic Failure

Background: Maternal mortality is defined as the death of any women while being pregnant or within 42 completed days of termination of pregnancy, irrespective of the duration or site of pregnancy, from any cause related to or aggravated by pregnancy, but not from accidental or incidental causes. Maternal mortality rate (MMR) is defined internationally as the maternal mortality rate per 100,000 live births. Even today 20% global maternal deaths occur in India. MMR in India is reduced by 77%, from 556 per 100,000 live births in 1990 to 130 per 100,000 live births in 2016 according to WHO & Millennium Development Goal (MDG) of 109 per 100,000 live births by 2015. Sustainable Development Goal (SDG3) goal of MMR less than 70 per 100,000 live births by year 2030 [21]. The main direct cause of maternal death in developing countries include haemorrhage, sepsis, obstructed labor and hypertensive disorders. These are mostly preventable trough regular antenatal checkup, proper diagnosis and management of labor complications. Aim and Objectives: 1.To assess the Maternal Mortality Rate(MMR) and causes of maternal mortality in given time period.2.Determine the relationship between maternal education, maternal age at first pregnancy and antenatal care attendance with maternal mortality. Material and Methods: A retrospective analysis of all maternal deaths (including their age at first pregnancy, education, antenatal visits) occurring in the Department of Obstetrics and Gynecology of a tertiary care hospital of eastern Maharashtra over a period of five years from January 2014 to December 2018. Data collected from death register of Medical Record Section of same institute. Statistical analysis: Data was expressed as count or percentage using MS Excel. Results: 21 maternal mortalities occurred during five year span with 35,205 live births giving MMR of 59.65 per 100,000 live birth. 42.86% mothers were of age group 20-24 yrs. Mothers with age between 15-34 yrs were also present. Out of all possible places of deaths 31 maternal mortalities occurred, most mortalities 67.74% occurred in hospital and 22.58% on the way to referral center. 52.38% mothers were educated upto 10th standard and 28.57% had done primary schooling. 42.86% mothers were with 4/>4 ANC visits and 4.76% with only one ANC visit. 38.09% mothers were with parity 1 and 9.52% with parity 4/>4. Major direct cause of death was hemorrhagic shock (47.61%) then eclampsia/pre-eclampsia (19.04%) and placenta previa (14.28%), sepsis (9.52%). Indirect causes were heart diseases (42.85%), anaemia (38.09%), respiratory diseases (14.28%) and sickle cell crisis (4.76%). Conclusion: As our MMR found to be 59.65 per 100,000 live births so we achieved the SDG goal 3. Staff should be trained for emergency cases and health services to pregnant women should be improved. Referral/ transportation system should be improved as most mortalities were preventable. Educating mothers and awareness camps should be arranged to strengthen motherhood and reduce MMR.

Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytosis. We investigated 148 biopsy specimens from 129 patients collected in the Kiel Pediatric Tumor Registry (KPTR) between 1965 and 2001. The clinical, histologic, and immunohistochemical characteristics of JXG were evaluated to gain more and deeper insights into the morphology and clinical behavior of JXG. Conventionally stained lesions were classified into the following morphologic subtypes: early JXG (EJXG), classic JXG (CJXG), transitional JXG (TJXG), or combined lesions with more than one basic pattern (combined JXG). Immunohistochemistry included antibodies against macrophages (Ki-M1P), S-100 protein, CD1a, and factor XIIIa (FXIIIa). Clinical data were obtained by means of a standardized questionnaire. The relative incidence of JXG in the KPTR is 0.52%. The male/female ratio was 1.4:1. The mean age was 22.4 months (median, 5 months; range, 0-244 months). A total of 34.5% of the cases of JXG were congenital, and 71.0% of the lesions were diagnosed within the first year of life. Most cases of cutaneous JXG were solitary (81.0%). Five cases (3.9%) presented with visceral (systemic) involvement. Histologically, CJXG was most frequent (47.2%), followed by EJXG (27.1%) and TJXG (16.0%). A total of 9.7% of the lesions represented combined JXG. Histiocytes, including giant cells, were positive for Ki-M1P (100%) and in most cases for FXIIIa (99%). The CD1a and S-100 protein reactions were generally negative. Clinical and follow-up data showed a generally favorable prognosis with a low relapse rate (7.0%) and even complete involution after incomplete resection. Only 1 of 5 patients with widespread congenital systemic disease died after 34 days. JXG is an uncommon, mostly cutaneous, and prognostically favorable histiocytic tumor of infancy. Simple tumor excision is the therapy for choice except in the very rare systemic JXG, in which multimodal chemotherapy is indicated.

The direct causes of death in Japanese patients with hypopituitarism remain unclear. In this study, the direct causes of death were investigated and compared between Japanese patients with hypopituitarism from a nation-wide autopsy database and an age- and gender-matched control population from national reports. Three hundred and ninety-one subjects with hypopituitarism who had died were selected from a nation-wide autopsy database (1984-1993). The ratios of each cause of death among the age- and gender-matched control population were derived from national reports. In subjects with hypopituitarism, an increased relative frequency of death from cerebrovascular diseases (male; 2.02 (95% confidence interval (CI) 1.45-2.82), female; 1.73 (95% CI 1.18-2.52)) was found. In particular, the relative frequency of death from cerebral hemorrhage was 4.60 (95% CI 2.95-7.17) in male and 4.80 (95% CI 2.90-7.94) in female subjects with hypopituitarism. Unexpectedly, a decreased relative frequency of death from all heart diseases (male; 0.439 (95% CI 0.277-0.696), female; 0.267 (95% CI 0.149-0.478)) was found in subjects with hypopituitarism, although there was no difference between subjects with hypopituitarism and controls in the frequency of death from ischemic heart disease. These results provide useful information for the long-term care of Japanese patients with hypopituitarism.

Evaluation of clinical harm associated with Omicron hospital-onset COVID-19 infection

Maternal mortality is a woman’s death during pregnancy or postpartum period due to direct or indirect causes. Its direct causes are related to obstetrical issues. The indirect cause of it refer to an underlying disease, i.e. aggravated during pregnancy and leads to death. A routine data-based study conducted at the Legal Medicine Organization of Tehran City, Iran, the autopsy findings of maternal mortalities during April 2017-2019 were reviewed. During the study period, 53 cases were autopsied of which 29 (54.7%) and 24 (45.3%) died due to direct and indirect causes, respectively. , The prevalent direct causes of death, were bleeding (22.6%), eclampsia/preeclampsia (13.2%), and ectopic pregnancy (5.7%). Frequent indirect causes were cardiac diseases (20.7%) and nervous system and infectious diseases (both: 7.5%). This study revealed the considerable share of indirect causes in maternal mortalities in Tehran. Moreover, we outlined the importance of early detection of non-obstetrical medical conditions during pregnancy care to reduce preventable maternal mortalities.

Juvenile xanthogranuloma is rare in children and there are limited data on its imaging features. To analyze the computed tomography (CT) and magnetic resonance imaging (MRI) features of juvenile xanthogranuloma in children. A retrospective review was performed of clinical and radiographic data of histologically confirmed juvenile xanthogranuloma between January 2009 and June 2020. Fourteen children (4 girls, 10 boys; age range: 1day to 13years, mean age: 73months) were included in thestudy: 4/14 had CT only, 5/14 had MRI only and 5/14 had CT and MRI. Sites of extracutaneous juvenile xanthogranuloma involvement included subcutaneous soft tissue (8/14), liver (2/14), lungs (2/14), kidney (2/14), nose (2/14), pancreas (1/14), central nervous system (1/14) and greater omentum (1/14), mainly manifested as single or multiple nodules or masses in different organs. On CT, the lesions mainly manifested as an iso-hypo density mass with mild or marked enhancement. On MRI, the lesions mainly manifested as slightly hyperintense on T1 and slightly hypointense on T2, with decreased diffusivity and homogeneous enhancement. Juvenile xanthogranuloma was not included in the imaging differential diagnosis in any case. Juvenile xanthogranuloma mainly manifests as single or multiple nodules or masses in different organs. Slight hyperintensity on T1 and slight hypointensity on T2 with decreased diffusivity and homogeneous enhancement are relatively characteristic imaging findings of juvenile xanthogranuloma. Combined with its typical skin lesions and imaging features, radiologists should include juvenile xanthogranuloma in the differential diagnosis when confronted with similar cases.

Child Abuse ReviewVolume 21, Issue 2 p. 77-80 EditorialFree Access Perspectives of Neglect First published: 26 March 2012 https://doi.org/10.1002/car.2208Citations: 3AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Neglect is a common, yet under-recognised form of child maltreatment (Glaser, 2002). In the UK, neglect is the most common category of abuse for children who are the subject of a child protection plan and its damaging effects on children and young people's health and wellbeing have been highlighted in two recent high-profile reports (Burgess et al., 2012; Daniel et al., 2011). Neglect is a significant public health issue, it is an underlying feature in many serious case reviews (reviews of non-accidental child deaths) and in extreme cases may be the direct cause of death (Brandon et al., 2010). Neglect rarely comes to light as a result of a specific incident (Hildyard and Wolfe, 2002) and often relies on practitioners making a judgement about the adequacy of ongoing care within a child and family context. Daniel (2005, p. 15) has described how a definition of neglect ‘must always be accompanied by careful delineation of the circumstances within which the child is living, the current and likely future physical and emotional impact and an analysis of the parental context’. It is the persistent failure to meet a child's needs and provide adequate care which is often significant, with its cumulative negative effects being detrimental to the child over a considerable period of time (Powell, 2007). ‘An underlying feature in many serious case reviews and in extreme cases may be the direct cause of death’ Yet, defining and identifying the presenting features of neglect are difficult and it is widely recognised as challenging for practitioners and researchers (Daniel et al., 2011; Taylor et al., 2005). The first paper in this issue describes how one borough council in the UK, Blackburn with Darwen, implemented a strategic approach to address the challenge of what constitutes adequate care. This council introduced multi-agency training workshops with 200 staff and appreciative working practices, to support the introduction of the Graded Care Profile (GCP) tool for supporting professional judgements about child neglect. Bernie Carter (2012) describes an evaluation study which took place alongside the initiative to investigate what was working well within child neglect practice and what changes occurred following implementation of the GCP. Despite initial reservations about the new model of working, over the course of the project there was a shift in the discourse of practitioners implementing the GCP from a focus on ‘neglect’ to a consideration of ‘quality of care’. Carter (2012, p. 95) reports how use of the GCP added ‘greater criticality’ to the professionals' observations of cases of neglect, ‘helped practitioners to have more positive conversations with parents’ and was helpful for less experienced practitioners. ‘A strategic approach to address the challenge of what constitutes adequate care’ In the second paper, June Slee (2012) outlines how in the Northern Territory of Australia failure to send children to school is classified as neglect by the Department of Health and Families. Slee describes how Indigenous children in Australia's remote Northern Territory have the poorest literacy and numeracy outcomes in Australia, as well as the highest rates of school non-attendance. In her informative paper, she contends that because authorities do not insist on children attending school and therefore fail to provide them with learning opportunities, that this is a form of systemic neglect. The paper examines some of the reasons for students' low school attendance, including the transient movement of Indigenous populations living in very remote areas and some children and families not seeing the value of education. Slee (2012, p. 111) argues that the key to addressing systemic neglect of school non-attendance and poor school performance is to attract and prepare Indigenous teachers to work at all levels, who can provide ‘culturally appropriate curricula’ as well as developing culturally responsive teaching amongst non-Indigenous staff. ‘Authorities do not insist on children attending school and therefore fail to provide them with learning opportunities’ Continuing the international focus on Australia, Shanti Raman and colleagues (2012) report on a research study which sought to examine whether front-line clinicians in general practice and hospital settings are equipped with the ability and confidence to address child abuse and neglect. Their survey, conducted in south west Sydney, involved general practitioners (GPs) and practice nurses working in primary care and emergency department (ED) doctors and nurses. These researchers found that while the majority of respondents understood their statutory and professional responsibilities to report suspected abuse or neglect, confidence levels in identifying abuse and neglect varied significantly between the two groups, with emergency doctors being the most consistent and practice nurses the least. The clinicians reported being most confident identifying physical abuse and neglect and less confident identifying emotional and sexual abuse. Of the 80 per cent of clinicians who had received some prior training in child abuse and neglect, 70 per cent of ED nurses, practice nurses and GPs reported their training to be inadequate, in comparison to 19 per cent of the ED doctors. These researchers conclude that there are significant differences between the confidence and reporting behaviour between front-line clinicians working in EDs and those working in primary care, and recommend targeted child protection training for front-line clinicians working in general practice. ‘Confidence levels in identifying abuse and neglect varied significantly between the two groups’ In a short report in this issue, Colette Selebo and colleagues (2012) describe an evaluation of a new specialist training programme developed in conjunction with the Royal College of Paediatrics and Child Health for paediatricians involved in legal processes. The two-day course on court skills training, delivered in a court setting, focused on family courts and criminal proceedings and explored the different orders available to the courts in child protection proceedings. The course provided participants with an opportunity to present evidence in mock trial sessions and to be cross-examined by two barristers. Evaluation feedback was received from 13 out of 15 students who participated in the course. All found the course highly relevant, with students being extremely positive about the value of preparing and presenting evidence in a mock trial. Following the two-day training course, seven participants had an opportunity to observe judges and barristers in a total of 14 cases representing a mixture of family, civil and criminal proceedings, including two cases of alleged neglect. The authors conclude that the success of the training is linked to the high staff-student ratio, and that such training is extremely important in improving the confidence of paediatricians when presenting evidence in court. ‘The course provided participants with an opportunity to present evidence in mock trial sessions’ Daryl Dugdale and Carol Wells (2012) in the final article of this issue address the subject of interprofessional training in the field of safeguarding children. These authors describe the development of a process and tool in which those responsible for inter-agency child protection training could be supported to audit and review the conditions in which training programmes were being delivered. The authors describe the three phases of the development of the audit tool, which included: (i) a review of the existing research and literature to identify key standards; (ii) adaptation of an existing model ‘to audit the conditions for delivery of effective inter-agency training’ (Dugdale and Wells, 2012, p. 143) and measure against existing standards; and (iii) testing of the tool with a number of Local Safeguarding Children Boards (LSCBs) and an interactive presentation with 70 interprofessional trainers who had an opportunity to try out the tool and provide feedback, which led to further development of the audit tool. The ‘Make a Difference’ tool is the outcome of the project. The authors report that the review tool is divided into three key areas of provision, participants and practice systems, which provide a framework to ‘demonstrate the training function's strengths and weakness’ (Dugdale and Wells, 2012, p. 148). Dugdale and Wells argue that the review tool's requirement to evidence responses results in a plan that can be owned and actions prioritised by the LSCB. ‘70 interprofessional trainers who had an opportunity to try out the tool and provide feedback, which led to further development of the audit tool’ The book review in this issue is by Jo Hebb (2012) who reviews Daniel Wilcox's authoritative and multi-authored practitioner's guide to the use of the polygraph in assessing, treating and supervising sex offenders. References Brandon M, Bailey S, Belderson P. 2010. Building on the Learning from Serious Case Reviews: A Two-year Analysis of Child Protection Database Notifications 2007–2009. Department for Education: London. Google Scholar Burgess C, Daniel B, Scott J, Mulley K, Derbyshire D, Downie M. 2012. Child neglect in 2011. First annual report on child neglect from Action for Children and the University of Stirling: Watford. Google Scholar Carter B. 2012. Developing and implementing an appreciative ‘quality of care’ approach to child neglect practice. Child Abuse Review 21: 81– 98. DOI: 10.1002/car.1198 Wiley Online LibraryWeb of Science®Google Scholar Daniel B. 2005. Introduction to issues for health and social care in neglect. In Child Neglect: Practice Issues for Health and Social Care, J Taylor, B Daniel, O Stevenson (eds). Jessica Kingsley Publishers: London. Google Scholar Daniel B, Taylor J, Scott J, Derbyshire D, Neilson D. 2011. Recognizing and Helping the Neglected Child. Evidence-Based Practice Assessment and Intervention. Jessica Kingsley Publishers: London. Google Scholar Dugdale D, Wells C. 2012. The Development of an Auditing tool to Support the Delivery of Inter-professional Training within the Field of Safeguarding Children. Child Abuse Review 21: 141– 150. DOI: 10.1002/car.1157 Wiley Online LibraryWeb of Science®Google Scholar Glaser D. 2002. Emotional abuse and neglect (psychological maltreatment): a conceptual frameowork. Child Abuse & Neglect 26: 697– 714. CrossrefPubMedWeb of Science®Google Scholar Gough D, Stanley N. 2007. Policy, Practice and Decision Making in Child Neglect. Child Abuse Review 16: 71– 73. DOI: 10.1002/car.985 Wiley Online LibraryGoogle Scholar Hebb J. 2012. Book review of The Use of the Polygraph in Assessing, Treating and Supervising Sex Offenders. A Practitioner's Guide edited by D. T. Wilcox. Child Abuse Review 21: 151– 152. DOI: 10.1002/car.1147 Wiley Online LibraryWeb of Science®Google Scholar Hildyard KL, Wolfe DA. 2002. Child neglect: developmental issues and outcomes. Child Abuse & Neglect 26: 679– 695. CrossrefPubMedWeb of Science®Google Scholar Powell C. 2007. Safeguarding Children and Young People. A Guide for Nurses and Midwives. Open University Press: Maidenhead. Google Scholar Raman S, Holdgate A, Torrens R. 2012. Are our Frontline Clinicians Equipped with the Ability and Confidence to Address Child Abuse and Neglect? Child Abuse Review 21: 114– 130. DOI: 10.1002/car1180 Wiley Online LibraryWeb of Science®Google Scholar Slee J. 2012. Addressing systemic neglect of young indigenous children's rights to attend school in the Northern Territory, Australia. Child Abuse Review 21: 99– 113. DOI: 10.1002/car1166 Wiley Online LibraryWeb of Science®Google Scholar Solebo C, Sidebotham P, Watson C. 2012. Preparing Paediatricians for Court: Evaluation of A Court Skills Training Programme. Child Abuse Review 21: 131– 140. DOI: 10.1002/car1161 Wiley Online LibraryWeb of Science®Google Scholar Taylor J, Daniel B, Stevenson O. 2005. Child Neglect: Practice Issues for Health and Social Care. Jessica Kingsley Publishers: London. Google Scholar Citing Literature Volume21, Issue2March/April 2012Pages 77-80 ReferencesRelatedInformation